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  • 1
    Electronic Resource
    Electronic Resource
    Inclusion Body Myositis: Clonal Expansions of Muscle-Infiltrating T Cells Persist Over Time (2003)
    Oxford, UK : Blackwell Science Ltd
    Scandinavian journal of immunology 58 (2003), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Inclusion body myositis (IBM) is a chronic inflammatory myopathy. The muscle histology is characterized by infiltration of T cells, which invade and apparently destroy muscle fibres. This study was performed to investigate whether predominant clones of muscle-infiltrating T cells are identical in different muscles and whether they persist over time in IBM. By reverse transcriptase-polymerase chain reaction, 25 T-cell receptor (TCR) variable β (Vβ) chain families and the complementarity-determining region 3 (CDR3) of the TCR were analysed in two different muscle biopsies of four patients with IBM. In two of the patients, the muscle biopsies were obtained from different muscles at one time point, whereas in two patients, the second biopsy was obtained 9 years after the first biopsy. T cells expressing predominant Vβ families were analysed for clonality by fragment length analysis of the CDR3. Predominant Vβ families were analysed by DNA sequencing to identify identical clones. Immunohistochemical staining of Vβ families was performed to study the distribution of T cells expressing identified predominant Vβ families. The muscle-infiltrating lymphocytes showed restricted expression of TCR Vβ families. DNA sequencing proved that clonally expanded T cells were identical in different muscles and persisted 9 years after the first biopsy. Immunohistochemical analysis with Vβ family-specific antibodies demonstrated the endomysial localization of these T cells in inflammatory cell infiltrates. Our results show that in IBM there is clonal restriction of TCR expression in muscle-infiltrating lymphocytes. Identical T-cell clones predominate in different muscles, and these clones persist for many years. These results indicate an important, continuous, antigen-driven inflammatory reaction in IBM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-3083.2003.01251.x
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  • 2
    Electronic Resource
    Electronic Resource
    Immunohistochemical demonstration of different muscle fibre types in paraffin sections (1989)
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 15 (1989), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.1989.tb01594.x
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  • 3
    Electronic Resource
    Electronic Resource
    Limited T-Cell Receptor V Gene Usage in Inclusion Body Myositis (1996)
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Scandinavian journal of immunology 43 (1996), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Inclusion body myositis (IBM) is a chronic, progressive inflammatory myopathy. The inflammatory infiltrates are dominated by T cells, which frequently invade muscle fibres. The present study was performed to characterize the usage of the variable (V)segment of the T-cell receptor of muscle infiltrating cells in IBM. Using the reverse transcriptase polymerase chain reaction (RT-PCR) technique the authors analysed the expression of 22 Vα and 24 Vβ families in muscle tissue from six patientswith IBM displaying intense inflammatory cell infiltration. The following Vα/Vβ families appeared in at least 50% of the patients: Vα1, 5, 7, 15, 16, 17, 20, 21, 22 and Vβ3, 5.2, 8, 12, 14, 22. In all patients Vα7, 16 and Vβ8 wereexpressed in muscle tissue. Furthermore, in two of the patients peripheral blood lymphocytes (PBL) were investigated in parallel. There was a restricted usage of Vα and Vβ families in muscle in comparison to PBL, indicating a selective homing orlocal proliferation of T lymphocytes in the inflammatory lesions in IBM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-3083.1996.d01-10.x
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  • 4
    Electronic Resource
    Electronic Resource
    Local T-Cell Proliferation and Differentiation in Inflammatory Myopathies (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 41 (1995), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Our objective was to investigate the patterns of proliferation and differentiation of infiltrating cells in inflammatory myopathies. Immunohistochemical staining was performed on muscle biopsy specimens from 18 patients with inclusion body myositis, polymyositis and dermatomyositis using monoclonal and polyclonal antibodies.An abundance of cells were TNF-α+ (4–8%), ICAM-1+ (7–65%). IFN-γ+ (3–6%), and Ki-67+ (4–8%). It was shown that 70% of the Ki-67+ cells were Ki-67+CD3+ cells. Very few mononuclear cells were IL-2R+. MHC-I expression was found on nearly all muscle fibres in all cases, while MHC-II expression was found on occasional muscle fibres in 1/3 of cases. Analysis of repeated biopsies from four IBM patients after prednisolone treatment showed no change in the proportions of TNF-α, ICAM-1, IFN-γ or Ki-67 positive cells. In inflammatory myopathies there is an intense proliferation and differentiation of inflammatory cells in situ, indicating a local stimulation of the inflammatory process.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1995.tb03587.x
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  • 5
    Electronic Resource
    Electronic Resource
    Low Levels of Mitochondrial Transcription Factor A in Mitochondrial DNA Depletion
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 200 (1994), S. 1374-1381 
    Details
    ISSN: 0006-291X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1006/bbrc.1994.1603
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  • 6
    Electronic Resource
    Electronic Resource
    Automatic sequencing of mitochondrial tRNA genes in patients with mitochondrial encephalomyopathy
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Molecular Basis of Disease 1226 (1994), S. 49-55 
    Details
    ISSN: 0925-4439
    Keywords: Mitochondria ; Myopathy ; encephalomyopathy ; mtDNA ; tRNA
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0925-4439(94)90058-2
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  • 7
    Electronic Resource
    Electronic Resource
    Muscle regeneration (1989)
    Springer
    Acta neuropathologica 78 (1989), S. 264-269 
    Details
    ISSN: 1432-0533
    Keywords: Skeletal muscle ; Regeneration ; Growth factors ; Growth hormone ; Rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Against the background of the importance of growth hormone (GH) for normal muscle growth, a study was performed to investigate whether lack of GH after hypophysectomy affects the cell proliferation and the local production of insulin-like growth factor-I (IGF-I) in the early stages of muscle regeneration in adult rats. The level of IGF-I in the serum of hypophysectomised rats was reduced to about 30% of that of controls. The incorporation of [methyl-3H]thymidine into the regenerating muscle showed a peak 6 days after the operation and then gradually declined to the end of the period of study 30 days after initiation of regeneration by ischemic necrosis. The DNA content rose to a maximum level after 6–8 days, and remained high after 30 days. There was no major difference in the incorporation of [3H]thymidine in regenerating muscle of hypophysectomised and control rats, but the DNA concentration in the regenerating muscles of hypophysectomised rats was significantly reduced after 30 days. There was a corresponding reduction in the number of nuclei per muscle fibre, indicating that hypophysectomy has a small effect on the cell proliferation during the early stages of muscle regeneration. Immunohistochemical demonstration of IGF-I in the regenerating muscle revealed the transient presence of immunoreactive material in satellite cells and myotubes after 6 to 8 days of regeneration but no immunoreactivity after 30 days. No obvious difference was observed between hypophysectomised and control rats, indicating that the endogenous production of IGF-I in regenerating skeletal muscle can occur independently of GH.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687756
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  • 8
    Electronic Resource
    Electronic Resource
    Mitochondrial encephalomyopathy (1987)
    Springer
    Acta neuropathologica 74 (1987), S. 287-293 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondrial encephalomyopathy ; Lactic acidosis ; Stroke-like episodes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report the clinical and autopsy findings in a young man of 18 with a chronic progressive disorder comprised of lactic acidosis, mental deterioration, and epileptic seizures which were sometimes accompanied by stroke-like episodes with transient hemiparesis and cortical blindness. He died of congestive heart failure. The autopsy showed lesions of the gray matter of the brain. Both the putamen and parieto-occipital cortex showed loss of neurons and proliferation of macrophages, astrocytes and vessels. There was marked loss of neurons in the inferior olives, and slight reduction of the number of Purkinje cells. Skeletal muscle studies revealed ragged-red fibres and structurally abnormal mitochondria. The heart was enlarged: accumulations of mitochondria occurred in the muscle fibers. The liver exhibited marked fatty degeneration. Biochemical analyses showed normal activities of pyruvate dehydrogenase in thrombocytes, pyruvate carboxylase in lymphocytes, biotinidase in serum as well as succinate dehydrogenase and cytochrome c oxidase. The features of this disorder differ in many respects from cases of mitochondrial encephalomyopathy previously reported and cannot be assigned to any specific disease entity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688194
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  • 9
    Electronic Resource
    Electronic Resource
    Peripheral nerve fibre degeneration in protein-deprived young rats (1982)
    Springer
    Acta neuropathologica 57 (1982), S. 1-6 
    Details
    ISSN: 1432-0533
    Keywords: Rat ; Malnutrition ; Protein deprivation ; Peripheral nervous system ; Ultrastructure ; Degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In a previous study the occurrence of nerve fibre degeneration with a distribution as in dying-back neuropathies was described in young rats subjected to severe protein deprivation (Oldfors 1981). In this study the ultrastructural appearance of the degeneration of the nerve fibres at different levels of the longitudinal tail nerves in severely protein-deprived rats has been investigated. Various structural changes were noted, the most common being bands of Büngner indistinguishable from those seen in Wallerian degeneration. In nerve fibres which were less severely affected the most common finding was shrinkage of the axon with concomitant folding of the myelin sheath. Other structural changes included axonal accumulation of 10 nm filaments or mitochondria and other cell organelles, areas of demyelination, and projections of axolemma and Schwann cell membrane into the axon. Signs of axonal regeneration occurred but were infrequent. The degenerative changes seen at various levels of the nerves support the view that the neuropathy is of distal axonal type, but the structural appearance differs from several of the toxic dying-back neuropathies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688871
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  • 10
    Electronic Resource
    Electronic Resource
    Neuropathology in Kearns-Sayre syndrome (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 541-546 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondria ; Central nervous system ; Skeletal muscle ; Pathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathological changes found at autopsy in a case of Kearns-Sayre syndrome are described. We have previously analyzed the respiratory chain function in isolated muscle mitochondria and also described a large deletion of muscle mitochondrial DNA (mtDNA) in this case. The neuropathological examination revealed prominent neuronal degeneration and gliosis of the basal ganglia and there were bilateral areas of softening and total loss of nerve cells in the lenticular nuclei. The pallidum and caudate nucleus disclosed accumulation of iron-containing pigment. The white matter in the cerebrum, brain stem and cerebellum showed widespread and focally accentuated spongy change due to splitting of myclin lamellae. It is suggested that deficiency of respiratory chain enzymes due to the mtDNA deletion is of pathogenetic importance in the development of the described changes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294616
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